Blood Transfusion in Sickle Cell Disease Leading to Posterior Reversible Encephalopathy Syndrome (PRES)

Abstract

Children with sickle cell disease have a very high risk of lifelong neurologic morbidity and mortality. Cerebrovascular accidents are a known complication in children with sickle cell disease. Posterior reversible encephalopathy syndrome is a constellation of acute neurologic findings increasingly recognized in pediatric critical care population with evidence of vasogenic edema on brain imaging possibly due to cerebral vascular endothelial cell dysfunction. This report, for the first time, describes a young adult with sickle cell disease who developed posterior reversible encephalopathy syndrome following blood transfusion.

Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease

“…Several proposed mechanisms of anemia‐induced cerebral vascular injury may be relevant to PRES including increased transcription of hypoxia inducible factor‐1 in the brain and increased shear stress that leads to increased nitric oxide production . These could lead to increased vascular permeability and VE.…”

“…Hemolysis also leads to nitric oxide scavenging and depletion that lead to vasculopathy. In addition, the platelet dysfunction and leukocyte activation lead to accumulation of proinflammatory cytokines such as TNF‐α and IL‐1 leading to endothelial damage . Figure summarizes the potential pathophysiologic mechanisms of PRES in SCD patients.…”

“…In Table , six cases were associated with ACS, 10 with exchange transfusion, five are in the setting of simple red cell transfusions, and sex predilection is not observed . The pathophysiology of ACS is known to be in part related to nitric oxide consumption by free Hb leading to vasoconstriction .…”

Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects

“…Serious adverse events, including death, have been reported from over-transfusion (Royal & Seeler, 1978;Serjeant, 2003;Raj et al, 2013). The patient's baseline Hb, transfusion status and %HbS should be taken into account when determining the target post-transfusion Hb in any given situation.…”

Neuroimaging findings in sickle cell disease

“…PRES in SCD has been reported in association with acute chest crisis, steroid use and overtransfusion. 26 In a recent cohort of 80 children with SCD, who presented with acute neurological symptoms, 10% demonstrated appearances consistent with PRES on neuroimaging. 27 In this study, PRES was associated with systemic hypertension and increased mortality, but interestingly not with pre-existing brain infarcts or intracranial vasculopathy.…”